NEUROPSYCHOLOGICAL DYSFUNCTION/NEUROIMAGING ABNORMALITIES IN ADULTS WITH SCD

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this research study is to determine the extent of memory and attention problems in adults with sickle cell disease. Neurologically intact adult patient's ages 21-55 years will be enrolled. That is adults who have not experienced a stroke or other brain damage. The study will also evaluate the link between these events or conditions and abnormal MRI. These findings will be compared to healthy people with similar backgrounds. The primary objective is to determine the extent of neurocognitive dysfunction in neurologically asymptomatic adult patients with sickle cell disease. The secondary objective to determine the association between neurocognitive dysfunction and imaging abnormalities. This is a cross-sectional investigation trial designed to compare neuropsychological function of adult neurologically normal Hb SS/SBo patients with matched-peer controls. A pilot intervention trial will randomize a subset of patients from the primary study to undergo either a chronic transfusion regimen or receive standard care alone, for six months. A subset of these subjects with WAIS III PIQ score greater than one standard deviation from the normative data (85) will be eligible for randomization in the pilot transfusion trial.